Congenital heart disease cannot be changed, but better tests and treatments are now available for children with these types of heart problems.

New therapies and technologies sized especially for children continue to improve the outcomes for children born with a congenital heart defect or who acquire a heart condition as they are growing up. In fact, according to the American Academy of Pediatrics, some 90 percent of children born with a heart defect survive to adulthood.

According to the American Heart Association, from 1994 to 2004, death rates for congenital heart defects declined more than 30 percent.

Dr. David Crowley is one of only a handful of pediatric cardiologists in New Hampshire. Based at Wentworth-Douglass Hospital in Dover, he also sees patients through Dartmouth-Hitchcock and at outreach clinics in Keene and in Wells, Maine. He has a close association with Children's Hospital in Boston, Mass.

"When you talk about pediatric heart disease and that of adults, they are worlds apart," Crowley said. "Most children's heart problems are congenital and may involve holes in the heart or unusual valve problems. Most babies are born perfectly healthy and only one in a hundred babies are at risk."

Crowley said heart problems in children can range from the simple to incredibly complex.

"Most common are holes, or narrow, leaky valves, problems that should not be there," he said.

Ventricular septal and atrial septal defects are correctable by surgery. Crowley said some babies have patent ductus arteriosus, caused by the valves that should close at birth and do not. The valves are unnecessary when babies begin breathing air.

"Atrial ventral canal defects happen when part of the heart is deformed and the child has one large valve instead of the usual two," he said. "This is a fairly common condition in children with Down syndrome."

All heart conditions sound terrifying to a new parent, but Crowley said advances in screening and techniques have improved the survival rate for babies from almost zero until the 1980s to 80 percent to 95 percent today. He said even the rate of sudden infant death syndrome, or SIDS, has been dramatically reduced by better testing and technology.

"It was common for babies to die because there were not a lot of good treatments," he said. "Now we have figured out a lot, including the safe use of a heart/lung machine to keep babies alive during surgery, and better surgical techniques for these small bodies. What used to be a rocky road has greatly improved.

"Today, I say having to break the news to parents is the single best and worst thing I do. It is the worst to say 'here is the bad news.' It is the best to say, 'here's what we can do to get through it.'"

Blue baby safeguard

The term blue baby refers to children born who are not receiving adequate levels of oxygen. Crowley helped pass a new law in New Hampshire last year called Parker's Law.

"It requires all birthing centers in the state to screen for critical heart conditions, especially for blue babies," Crowley said. "It is a simple test of O2 stats (oxygen). I am delighted with this law because it catches conditions in babies who do not appear sick but are."

Crowley said the test can save babies who might get into extreme trouble after leaving the hospital. The test is simple and does not hurt.

"It's a little sensor placed on a finger and a toe that measures that oxygen saturation level," he said.

The test is done after 24 hours for reliability. Crowley said circulation levels change 24 hours after birth as the baby's lungs fully expand.

Fetal monitoring for heart problems is done routinely now, usually on the referral of an obstetrician who suspects a problem. Crowley said problems are caught much earlier.

"Most of the time we do primarily monitoring, for fetal distress, or for excess fluid retention in the fetus," he said. "However, there are cases when we will intervene in utero. If a baby is having arrhythmia, we can give the mom medication to regulate the heart rate. If a mom has a condition such as lupus, we can use steroids for a slow heart rate. Occasionally, we will do surgical interventions, but that is high risk so it's used only when absolutely needed."

Still, knowing what is coming does help parents prepare.

"You can't measure peace of mind," Crowley said. "But knowing ahead of time gives parents time to prepare and us, as physicians, time to plan our best course of action."